Executive performance on the preschool executive task assessment in children with sickle cell anemia and matched controls

ABSTRACT

Executive deficits are commonly reported in children with sickle cell anemia. Earlier identification of executive deficits would give more scope for intervention, but this cognitive domain has not been routinely investigated due to a lack of age-appropriate tasks normed for preschool children. In particular, information relating to patient performance on an executive task that reflects an everyday activity in the classroom could provide important insight and practical recommendations for the classroom teacher at this key developmental juncture as they enter the academic domain. The performance of 22 children with sickle cell anemia was compared to 24 matched control children on the Preschool Executive Task Assessment. Findings reveal that children with sickle cell anemia are performing poorer than their matched peers on this multi-step assessment. In particular, children with sickle cell anemia required more structured support to shift focus after a completed step, as reflected by poorer scores in the quantitative Sequencing and Completion domains. They also required more support to stay on task, as seen by poorer ratings in the qualitative Distractibility domain.

Abbreviations:PETA: Preschool Executive Task Assessment; SCA: Sickle Cell Anemia; EF: Executive Functioning.

KEYWORDS:

• Executive function
• neuropsychological assessment
• preschool
• sickle cell disease
• sickle cell anemia
• neurodevelopmental disorders

Acknowledgments

The authors would like to thank the families who participated in this research. This research was funded by the Child Health Research Charitable Incorporated Organization and supported by the National Institute for Health Research Biomedical Research Centre at Great Ormond Street Hospital for Children NHS Foundation Trust and University College London. MdH was supported by Great Ormond Street Hospital Children’s Charity. PETA Task materials, including training video and manual, are available by request to first author.

Disclosure statement

The authors have no conflict of interest.

Additional information

Funding

This work was supported by the Child Health Research Charitable Incorporated Organization; National Institute for Health Research Biomedical Research Centre at Great Ormond Street Hospital for Children NHS Foundation Trust; University College London; and Great Ormond Street Hospital Children’s Charity.